Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes

Clin Liver Dis. 2022 Aug;26(3):341-354. doi: 10.1016/j.cld.2022.03.001. Epub 2022 Jun 25.


Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow progression after diagnosis. Variable contribution of genetic, immune, and environmental factors contributes to disease heterogeneity among patients with biliary atresia. Developing a deeper understanding of the disease mechanism will help to develop targeted medical therapies and improve patient outcomes.

Keywords: Biliary atresia; Neonatal cholestasis; Pediatric liver transplantation; Transplant outcomes.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Biliary Atresia* / diagnosis
  • Biliary Atresia* / genetics
  • Biliary Atresia* / therapy
  • Child
  • Humans
  • Infant
  • Liver Transplantation*